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85 Cards in this Set
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Lower motor neuron lesions only, due to destruction of anterior horns
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Poliomyelitis and Werdnig-Hoffman disease
flaccid paralysis!! |
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Werdnig-Hoffman disease
(infantile spinal muscular atrophy) |
AR inheritance; presents at birth as a 'floppy-baby', tongue fasciculations; median age of death is 7 months
associated with degeneration of anterior horns LMN involvement only |
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Polio nerve stuff
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follows infection with poliovirus
LMN signs associated with degeneration of anterior horns |
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Poliomyelitis
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caused by poliovirus, which is transmitted by the fecal-oral route
replicates in the oropharynx and small intestine before spreading through the bloodstream to the CNS, where it leads to the destruction of cells in the anterior horn of the spinal cord...LMN destruction |
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Symptoms of poliomyelitis
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malaise, HA, fever, nausea, abdominal pain, sore throat
Signs of LMN lesions: muscle weakness and atrophy, fasciculations, fibrillation and hyporeflexia |
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Findings of poliomyelitis
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CSF with lymphocytic pleocytosis with slight elevation of protein (with no change in CSF glucose)
virus recovered from stool or throat |
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Spinal cord lesion of MS
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mostly white matter of cervical region
random and asymmetric lesions, due to demyelination scanning speech, intention tremor, nystagmus |
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General with MS
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increased prevalence with increase distance from the equator
periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with preservation of axons many patients have a relapsing-remitting course most often affects women in their 20s and 30s; more common in whites |
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Increase protein (IgG) in CSF
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MS
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Patients with MS present with...
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optic neuritis (sudden loss of vision), MLF syndrome (internuclear ophthalmoplegia), hemiparesis, hemisensory symptoms, or bladder/bowel incontinence
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Classic triad of MS
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SIN!!
S: scanning speech I: intention tremor, incontinence, internuclear opthalmoplegia N: nystagmus |
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Treatment of MS
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beta-interferon or immunosuppressant therapy
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ALS
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combined upper and lower motor neuron deficits with NO sensory deficits
BOTH UMN and LMN signs |
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Can be caused by a defect in superoxide dismutase 1 (SOD 1)
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ALS
aka. Lou Gehrig's disease |
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Complete occlusion of anterior spinal artery
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spares dorsal columns and tract of Lissauer
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Tabes dorsalis spinal lesion
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tertiary syphilis
degeneration of dorsal roots and dorsal columns; impaired proprioception, locomotor ataxia |
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Tabes dorsalis
(even more!) |
degeneration of dorsal columns and dorsal roots due to tertiary syphilis, resulting in impaired proprioception and locomotor ataxia
associated with Charcot's joints (degeneration of joint surfaces from loss of proprioception), shooting (lightning pain), Argyll Robertson pupils (reactive to accomodation but not to light) and absence of DTRs |
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Syringomyelia spinal lesion
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crossing fibers of spinothalamic tract are damaged
bilateral loss of pain and temperature sensation |
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More on syringomyelia
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enlargement of central canal of spinal cord
crossing fibers of spinothalamic tract are damaged bilateral loss of pain and temperature sensation in the upper extremities with preservation of touch sensation often presents in patients with Arnol-Chiari malformation (congenital herniation of cerebellar tonsil through foramen magnum) Most common at C8-T1 |
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Vitamin B12 neuropathy and Friedreich's ataxia spinal lesions
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demylination of dorsal columns, lateral corticospinal tracts, and spinocerebellar tracts; ataxic gait, hyperreflexia, impaired position and vibration sense
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Brown-Sequard syndrome
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hemisection of spinal cord
Findings: 1. ipsilateral UMN signs (corticospinal tract) below lesion 2. ipsilateral loss of tactile, vibration, proprioception sense (dorsal column) below lesion 3. contralateral pain and temperature loss (spinothalamic tract) below lesion 4. ipsilateral loss of all sensation AT level of lesion 5. LMN signs (flaccid paralysis) AT level of lesion If the lesion occurs above T1, presents with Horner's syndrome |
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Horner's syndrome triad
(associated with lesion of spinal cord above T1) |
1. ptosis (slight drooping of eyelid)
2. anhidrosis (absence of sweating) and flushing (rubor) of affected side of face 3. Miosis (pupil constriction) |
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Alzheimer's disease
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most common cause of dementia in the elderly
senile plaques (extracellular, with beta-amyloid core) neurofibrillary tangles (intracellular, abnormally phosphorylated tau protein, tangles correlate with degree of dementia) diffuse cortical atrophy |
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Familial form of Alzheimer's is associated with...
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10%: genes on chromosomes 1, 14, 19 (APOE4 allele) and chromosome 21 (p-APP gene)
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2nd most common cause of dementia in elderly
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multi-infarct dementia
may cause amyloid angiopathy-> intracranial hemorrhage |
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Pick's disease
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FRONTOTEMPORAL DEMENTIA
frontotemporal lobe atrophy, dementia, aphasia, parkinsonian aspects, associated with Pick bodies (intracellular tau protein) |
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Lewy body dementia
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parkinsonism with dementia and hallucinations
caused by alpha-synuclein defect |
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Creutzfelt-Jakob disease
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prions!!
rapidly progressive (weeks to months) dementia with myoclonus, spongiform cortex; associated with prions |
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Huntington's disease
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AD!!
chorea, dementia, atrophy of caudate nucleus (loss of GABAergic neurons) triplet repeat defect causes genetic anticipation degeneration of caudate leads to enlarged lateral ventricles on CT |
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Chromosome 4
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Huntingtons!!
expansion of CAG repeats CAG: caudate loses ACh and GABA |
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Parkinson's disease
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associated with Lewy Bodies (composed of alpha synuclein) and depigmentation of the substantia nigra pars compacta (loss of dopaminergic neurons)
rare cases have been linked to exposure of MPTP, a contaminant of illicit street drugs |
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TRAP, with regards to Parkinson's disease
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Tremor
cogwheel Rigidity Akinesia Postural instability (you are TRAPped in your body) |
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Spinocerebellar degenerative diseases
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olivopontocerebellar atrophy and Friedreich's ataxia
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Progressive multifocal leukoencephalopathy (PML)
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most often the cause is the JC polyoma type of papovavirus, which preferentially infects oligodendrocytes, thus causing demyelination
seen in 2-4% of AIDS patients (reactivation of latent viral infection) |
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Acute disseminated (postinfectious) encephalomyelitis
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disease follows viral illnesses wuch as measles, mumps, rubella and chickenpox
widespread demyelination |
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Metachromatic leukodystrophy
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AR lysosomal storage disease
arylsulfatase A deficiency |
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Guillan Barre syndrome
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inflammation and demyelination of peripheral nerves and motor fibers of ventral roots (sensory effect less severe than motor)
symmetric ascending muscle weakness beginning in distal lower extremities facial paralysis in 50% of cases autonomic function may be severely affected (like cardiac irregularities, hypertension or hypotension) almost all patients survive; the majority recover completely after weeks to months |
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Guillan Barre is associated with...
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infections!!
autoimmune attack of peripheral myelin due to molecular mimicry (like Campylobacter jejuni or herpes infection), inoculations and stress...but no definitive link to pathogens (must give respiratory support until recovery...additional treatment includes plasmapheresis and IV immune globulins) |
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Albuminocytologic dissociation
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elevated CSF protein with normal cell count
elevated protein will lead to papilledema |
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Communicating hydrocephalus
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normal pressure hydrocephalus
enlarged ventricles with normal opening pressure on lumbar puncture Classic triad: dementia, gait problems, urinary incontinence caused by impaired absorption of CSF by arachnoid granulations |
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Obstructive (non-communicating) hydrocephalus
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caused by structural blockage of CSF circulation within the ventricular system (like stenosis of the aqueduct of Sylvius)
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Sturge Weber syndrome
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congenital disorder with port-wine stains and ipsilateral leptomeningeal angioma (benign tumors made of small blood vessels)
can cause glaucoma, seizures, hemiparesis, and mental retardation |
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Tuberous sclerosis
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hamartomas in the CNS, skin, organs; cardiac RHABDOMYOMA, renal angiomyolipoma
subependymal giant cell astrocytoma, MR, seizures, ash leaf spots, sebaceous adenoma, shagreen patch |
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Neurofibromatosis
(von Recklinghausen disease) |
cafe-au-lait spots, Lisch nodules (iris), neurofibromas in skin
multiple neurofibromas in skin, schwannomas of the VIIIth nerve Skeletal disorders: scoliosis adn bone cysts...increased incidence of other tumors (NF1 gene...) |
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von Hippel-Lindau disease
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AD disorder with cavernous hemangiomas in skin, mucosa, organs
RENAL CELL CARCINOMA, hemangioblastoma in retina, brain stem, cerebellum hemangioblastoma or cavernous hemangioma; remarkably increased incidence of RCC!!! short arm of chromosome 3 |
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Tay-Sachs disease
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most common form of gangliosidosis and occurs primarily in those of Ashkenazi Jewish peeps
deficiency of hexosaminidase A...accumulates GM2 ganglioside CNS degeneration, severe mental and motor deterioration, blindness, characteristic cherry-red spot |
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Gaucher disease
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disorder of lipid metabolism
deficiency of glucocerebrosidase, accumulates glucocerebroside Gaucher cells: wrinkled tissue paper cytoplasmic appearance |
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most common primary brain tumor
prognosis grave (less than 1 year) cerebral hemispheres can cross corpus callosum stain astrocytes for GFAP |
glioblastoma multiforme
(grade IV astrocytoma) |
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'pseudopalisading' plemorphic tumor cells - border central area of necrosis and hemorrhage
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glioblastoma multiforme
(grade IV astrocytoma) |
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2nd most commone primary brain tumor
most often occurs in convexities of hemispheres and parasagittal region arises from arachnoid cells external to brain resectable |
Meningioma
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spindle cells concentrically arranged in a WHORLED PATTERN
PSAMMOMA bodies (laminated calcifications) |
Meningioma
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3rd most common primary brain tumor
Schwann cell origin often localized to CN VIII --> acoustic schwannoma resectable! |
Schwannoma
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bilateral schwannoma found in neurofibromatosis type 2
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schwannoma
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Relatively rare, slow growing
most often in frontal lobes chicken-wire capillary pattern |
Oligodendroglioma
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'fried egg' appearance
round nuclei with clear cytoplasm often calcified in oligodendroglioma |
Oligodendroglioma
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Prolactin secreting is most common form
bitemporal hemianopia (due to pressure on optic chiasm) and hyper or hypopituitarism are sequelae |
pituitary adenoma
(Rathke's pouch) |
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Usually well circumscribed
in children, most often found in posterior fossa benign and good prognosis |
pilocytic (low-grade) astrocytoma
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rosenthal fibers - eosinophilic, corkscrew fibers
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Pilocytic (low grade) astrocytoma
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Highly malignant cerebellar tumor
a form of primitive neuroectodermal tumor (PNET) can compress 4th ventricle, causing hydrocephalus |
medulloblastoma
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Rosettes or perivascular pseudorosette pattern of cells
radiosensitive |
Medullablastoma
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Ependymal cell tumors most commonly found in fourth ventricle
can cause hydrocephalus poor prognosis |
Ependymoma
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characteristic perivascular pseudorosettes; rod-shaped blepharoplasts (basal-ciliary bodies) found near nucleus
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ependymoma
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Most often cerebellar
associated with von Hippel-Lindau syndrome when found with retinal angiomas can produce EPO which leads to secondary polycythemia |
hemangioblastoma
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foamy cells and high vascularity are characteristic
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hemangioblastoma
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benign childhood tumor, confused with pituitary adenoma (can also cause bilateral hemianopia)
most common childhood supratentorial tumor |
Craniopharyngioma
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Derived from Rathke's pouch remnants
Calcificaiton is common (tooth enamel-like) |
craniopharyngioma
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Arnold Chiari
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small posterior fossa
downward displacement of cerebellum, medulla deformity; associated with tonsillar herniation |
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Chiari I
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low lying cerebellum obstructs CSF flow and compresses medulla
cerebellar tonsils descend through foramen magnum frequently asymptomatic; correctable with surgery |
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Chiari II
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cerebellar vermis and medulla descend through foramen magnum
fatal!!! |
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Dandy Walker
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large posterior fossa; absent cerebellum with cyst in its place
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unilateral lesion of cerebellum
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patient tends to fall TOWARD side of lesion
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CN X lesion
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uvula deviates AWAY from lesion
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CN XI lesion
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weakness turning head to contralateral side of lesion
shoulder droop on side of lesion |
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UMN lesion
(facial lesion) |
contralateral paralysis of lower face only
(lesion between motor cortex or connection between cortex and facial nucleus) ...corticobulbar tract...UMN lesion |
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LMN lesion
(facial nucleus) |
ipsilateral paralysis of upper and lower face
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Bell's Palsy
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complete destruction of the facial nucleus itself or its branchial efferent fibers (facial nerve proper)
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What happens in Bell's Palsy
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peripheral ipsilateral facial paralysis with inability to close eye on involved side
only lower face is affected, since upper face has contralateral and ipsilateral innervation by CN VII |
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Causes of Bell's palsy
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can occur idiopathically, gradual recovery in most cases
seen as a complication in AIDS, Lyme disease, Sarcoidosis, Tumors and Diabetes |
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Cingulate (subfalcine) herniation
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under falx cerebri
superior frontal or parietal mass lesions |
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Transtentorial herniation
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central!
downward herniation |
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Uncal herniation
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medial temporal lobe herniation
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Herniation into the foramen magnum
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cerebellar tonsillar herniation
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Uncal herniation signs
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1. ipsilateral dilated pupil/ptosis (stretching of CN III)
2. contralateral homonymous hemianopia (compression of ipsilateral posterior cerebral artery) 3. ipsilateral paresis (Kernohan's notch) 4. Duret hemorrhages |
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Kernohan's notch
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in uncal herniation, you get ipsilateral paresis...due to compression of contralateral crus cerebri (Kernohan's notch)
this is a 'false localizing' sign |
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Duret hemorrhages
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paramedian artery rupture
secondary to increased ICP caudal displacement of the brain stem |
